Rationale: Precapillary pulmonary hypertension (PH) is a rare and largely unrecognized complication of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF). Objectives: To describe characteristics and outcomes of MPN-associated PH. Methods: We report clinical, functional, and hemodynamic characteristics, classification, and outcomes of patients with PV, ET, or primary MF in the French PH registry. Measurements and Main Results: Ninety patients with MPN (42 PV, 35 ET, 13 primary MF) presented with precapillary PH with severe hemodynamic impairment, with a median mean pulmonary arterial pressure and pulmonary vascular resistance of 42 mm Hg and 6.7 Wood units, respectively, and impaired clinical conditions, with 71% in New York Heart Association functional classes III/IV and having a median 6-minute-walk distance of 310 m. Half of the patients were diagnosed with chronic thromboembolic PH (CTEPH); the other half were considered to have group 5 PH. MF was preferentially associated with group 5 PH, whereas PV and ET were generally related to CTEPH. Proximal lesions were diagnosed in half of the patients with CTEPH. Thromboendarterectomy was performed in 18 selected patients with high risk of complications (5 early deaths). Overall survival at 1, 3, and 5 years was 67%, 50%, and 34% in group 5 PH and 81%, 66%, and 42% in CTEPH, respectively. Conclusions: PH is a life-threatening condition potentially occurring in MPN. There are multiple mechanisms, with equal diagnoses of CTEPH and group 5 PH. Physicians should be aware that PH strongly affects the burden of patients with MPN, especially in group 5 PH, with unknown pathophysiological mechanisms.

World Health Organization (WHO) group 5 pulmonary hypertension (PH) entails a heterogeneous group of disorders that may cause PH by unclear and/or multiple mechanisms. In particular, group 5 includes PH caused by hematologic disorders, systemic diseases, metabolic disorders, chronic renal failure, and disorders leading to pulmonary vascular occlusion or compression. This article discusses common pathogenic mechanisms leading to group 5 PH, followed by a detailed overview of epidemiology, pathogenesis, and disease-specific management of the individual group 5 conditions. Off-label use of vasomodulatory therapies, typically indicated for pulmonary arterial hypertension (WHO group 1 PH), in group 5 conditions is also discussed.

Malignant and nonmalignant disorders may affect the liver, causing signs and symptoms ranging from mild increases of liver tests to fulminant hepatic failure. This article discusses the most common hematologic and oncologic disorders and their effect on the liver. The section on nonmalignant hematologic disorders includes the anemias, paroxysmal nocturnal hemoglobinuria, disseminated intravascular coagulation, malaria, Banti syndrome, the porphyrias, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Malignant hematologic conditions include leukemias, lymphomas, and myeloproliferative disorders. Other conditions causing portal hypertension and hepatic metastases are also discussed. The most commonly encountered hepatic manifestations of hematologic and oncologic disorders are reviewed.

Increased incidence of pulmonary hypertension (PH) has been reported in patients with chronic myeloproliferative disorders. The exact incidence of PH in essential thrombocythemia (ET) is unknown. Most of the reported literature consists of case reports or small studies. We designed this study to asses the incidence of PH in patients with ET and reactive thrombocytosis. Previously or newly diagnosed 46 patients with ET, and 40 patients with reactive thrombocytosis secondary to iron deficiency anemia were found to be eligible for this study. Diagnosis of PH was established via transthoracic echocardiography. PH was found in 22 (47.8%) out of 46 patients with ET. Seven patients with PH were newly diagnosed ET, 5 patients with PH were in low, and the other patients with PH were in intermediate or high risk category. We found statistically significant difference in terms of platelet counts between ET patients with PH and without PH (p = 0.027). None of the patients with reactive thrombocytosis had PH. In conclusion, PH appears to be common in patients with ET. Therefore, all patients with ET should be evaluated for PH. Larger and prospective studies are required to clarify the long-term impact of PH on the survival of these patients. Future studies are also needed to determine whether cytoreductive treatment and aspirin prevent the development of PH, and to determine the effects of cytoreductive treatments and aspirin on the prognosis of PH. The effect of PH on ET prognosis should also be determined in low risk ET patients.

We describe a patient with myelofibrosis, giant splenomegaly, and pulmonary hypertension related to increased intra-abdominal pressure. Focusing on alterations in hemodynamic studies, we conclude that in patients with myelofibrosis, dyspnea, and hypoxemia, the measurement of intra-abdominal pressure should be included in the initial evaluation. It is an inexpensive, non-invasive diagnostic tool that can provide crucial information about the cause of dyspnea and disclose the pathogenetic link between massive splenomegaly and pulmonary compromise in myelofibrosis.